| Identification | Back Directory | [Name]
1-Propanaminium, 3-carboxy-N,N,N-trimethyl-2-(2-methyl-1-oxobutoxy)-, inner salt, (2R)- | [CAS]
256928-75-3 | [Synonyms]
2-Methylbutyryl-L-carnitine >=97.0% (TLC)
3-((2-Methylbutanoyl)oxy)-4-(trimethylammonio)butanoate
1-Propanaminium, 3-carboxy-N,N,N-trimethyl-2-(2-methyl-1-oxobutoxy)-, inner salt, (2R)- | [Molecular Formula]
C12H23NO4 | [MDL Number]
MFCD11975299 | [MOL File]
256928-75-3.mol | [Molecular Weight]
245.32 |
| Hazard Information | Back Directory | [Uses]
2-Methylbutyrylcarnitineis was detected in a study of maternal liver metabolic response to vitamin D deficiency. | [Definition]
ChEBI: 2-methylbutyrylcarnitine is a C5-acylcarnitine having 2-methylbutyryl as the acyl substituent. It has a role as a human metabolite. It is functionally related to a 2-methylbutyric acid. | [Biological Activity]
2-Methylbutyryl-L-carnitine is not usually detected in normal individuals. The elevation of 2-methylbutyrylcarnitine suggests a deficiency of a dehydrogenase specific for isobutyryl-CoAimportant in the differential diagnosis of branched chain organic acidurias by analysis of urinary organic acids and acylcarnitines in plasma or dried blood spots. |
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